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Objectives: To identify the risk factors and clinical manifestations associated with skull base osteomyelitis., Methods: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data from July 2013 to October 2020 related to adult patients of either who presented with base osteomyelitis. All relevant data was retrieved from hospital records. Data was analysed using SPSS 16., Results: Of the 56 patients with a mean age of 58.12±17.79 years, 39 (69.6%) were males. Diabetes was the most common comorbidity (n 39, 69.6%). The majority of patients had chronic sinusitis (n 34,60.7%) otitis media (n 20, 35.7%) or head and neck surgery (n 18, 32.1%) within the preceding 2 years. The mean duration between onset of symptoms and presentation to healthcare facility was 12±13.96 weeks. The most common presenting symptom was headache (n 38, 68%). The majority of patients (n 34 ,59.6 %) had fungal organisms isolated from tissue culture. Cranial nerve palsies were present in 44 (77%) patients. Out of 56 patients, 22(39.2%) were lost to follow and overall mortality was high (n 11, 19.6%). Sepsis was found to be associated with increased mortality (p=0.008)., Conclusions: Skull base osteomyelitis was associated with significant morbidity and mortality, and posed significant diagnostic and therapeutic challenges.

Cases of herpes zoster ophthalmicus (HZO) complicated by bilateral ophthalmoplegia are rare, and no cases of bilateral third, fourth, or sixth cranial nerve palsies have been reported. Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a rare complication of HZO. We herein report an 80-year-old Japanese woman with right-sided HZO complicated by meningoencephalitis and discuss the pathogenesis of this condition. She developed bilateral third, fourth, and sixth cranial nerve palsies and SIADH almost simultaneously during treatment for HZO. The bilateral cranial palsy spontaneously resolved within a few months.

Background and Purpose: Cervical artery dissection (CAD) represents a leading cause of unilateral lower cranial nerve IX-XII palsy, known as Collet-Sicard syndrome (CSS). High-resolution magnetic resonance imaging (HR-MRI) is widely used in the evaluation of patients with CAD, providing information regarding vessel wall abnormalities and intraluminal thrombus., Methods: We present a patient with palsy of multiple lower cranial nerves in the context of CSS, attributed to unilateral spontaneous internal carotid artery dissection., Results: We describe a 68-year-old man with unremarkable previous history, who presented with subacute, gradually worsening dysphagia and hoarse voice. Clinical examination revealed right-sided palsy of cranial nerves IX-XII. Three-dimensional fat-saturated black-blood T1-weighted high-resolution vessel wall imaging disclosed spontaneous dissection with intramural hematoma along the distal right internal carotid artery. Neck MRI showed inward displacement of right aryepiglottic fold, right pyriform sinus dilatation, and right true vocal cord in middle position, indicative of right vagus nerve palsy, atrophy of right trapezius and sternocleidomastoid muscles, due to right spinal accessory nerve palsy, and unilateral tongue atrophy with fatty infiltration, characteristic for right hypoglossal nerve palsy., Conclusions: This case highlights the utility of high-resolution vessel wall imaging and especially fat-saturated T1-weighted black-blood SPACE (sampling perfection with application-optimized contrast using different flip-angle evolutions) sequences in the accurate diagnosis of CAD, revealing the characteristic mural hematoma and intimal flap. HR-MRI is also valuable in the recognition of indirect signs of lower cranial nerve compression., (© 2024 The Author(s). European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Purpose: Cranial Nerve Neuropathies (CNNs) often accompany Cavernous Sinus Meningioma (CSM), for which Stereotactic Radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSR) are established treatments. This study assesses CNNs recovery in CSM patients treated with LINAC, offering insight into treatment effectiveness., Methods: This study was conducted on 128 patients with CSM treated with LINAC-based SRS/FSR between 2005 and 2020 at a single institution. 46 patients presented with CNNs. The study analyzed patients' demographics, clinical parameters, SRS/FSR treatment characteristics, post-treatment CNNs recovery duration, status, and radiological control on their last follow-up., Results: The median follow-up duration was 53.4 months. Patients were treated with SRS (n = 25) or FSR (n = 21). The mean pretreatment tumor volume was 9.5 cc decreasing to a mean end-of-follow-up tumor volume was 5.1 cc. Radiological tumor control was achieved in all cases. CNN recovery was observed in 80.4% of patients, with specific nerve recoveries documented as follows: extra-ocular nerves (43.2%), trigeminal nerve (32.4%), and optic nerve (10.8%). A higher CNNs recovery rate was associated with a smaller pre-treatment tumor volume (p < 0.001), and the median time-to-improvement was 3.7 months. Patients with tumor volumes exceeding 6.8 cc and those treated with FSR exhibited prolonged time-to-improvement (P < 0.03 and P < 0.04 respectively)., Conclusions: This study suggests that SRS/FSR for CSM provides good and sustainable CNNs recovery outcomes with excellent long-term radiological control. A higher CNNs recovery rate was associated with a smaller pre-treatment tumor volume. while shorter time-to-improvement was identified in patients treated with SRS compared to FSR, particularly in those with small pre-treatment tumor volume., (© 2024. The Author(s).)

Objectives: Cranial nerve (CN) involvement is not a common feature of typical chronic inflammatory demyelinating polyneuropathy (CIDP). Patients with acute presentation of CN palsy in CIDP may be misdiagnosed and treated as other pathologies., Methods: We report a patient with multiple cranial neuropathies at the onset of CIDP in detail. In addition, we reviewed a large cohort of patients with CN involvement in CIDP and summarized their characteristics and clinical findings., Results: We presented a 28-year-old woman who presented with progressive weakness and involvement of CN III, VII, X, XII in the subacute phase who was diagnosed as CIDP and was treated accordingly. A scoping review of the literature resulted in a total of 59 patients with available patient-level data [61.2% men, median age of 32 (Q1-Q3; 20-51.5) years]. CN impairment was present in the acute phase of the polyneuropathy in 10 out of 43 patients (23.3%), while it took a median of 7.7 [Q1-Q3; 3-13] years for other patients to present CN palsy. Sensitivity analysis did not reveal any difference among patients with acute-phase presentation of CN symptoms (N = 11) compared with those with delayed CN palsy (N = 33) in terms of demographics, patterns of CN involvement, associated diminished sensorimotor findings, or relapse. However, patients with acute presentation of CN palsy underwent plasmapheresis approximately 4 times more than those with delayed CN presentations (45.5% vs. 12.1%, P = 0.02)., Conclusion: In this case presentation and review study, we observed that in one-fourth of patients with CIDP and CN neuropathy, CN involvement occurred in the acute phase. This finding indicates the necessity of considering CIDP among differential diagnoses of patients with CN involvement and polyneuropathies., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: Skull base tumors, can cause oculomotor dysfunction, presenting a management challenge given their proximity to cranial nerves. This study investigated the oculomotor outcomes in patients with skull base tumors presenting cranial nerve palsy due to tumor compression and aimed to identify associated factors., Methods: This retrospective observational cohort study enrolled patients diagnosed with primary skull base tumors who exhibited cranial nerve palsy due to tumor compression, confirmed by magnetic resonance imaging treated at Asan Medical Center between January 2011 and December 2022. Patients were assessed for oculomotor function pre- and post-treatment, and categorized into recovery and non-recovery groups based on outcomes. Factors associated with oculomotor outcomes were also analyzed., Results: Fifty-six patients were enrolled, with the majority (n = 37, 66.1%) demonstrating recovery in oculomotor function post-treatment. The duration from symptom onset to treatment initiation was short in the recovery group, suggesting that early treatment may contribute to improved oculomotor outcomes. The type of tumor was significantly associated with oculomotor outcomes, with patients with pituitary adenoma exhibiting better outcomes. In the recovery group, 19/37 (51.4%) patients underwent surgical resection alone. In contrast, in the non-recovery group, 17/19 (89.5%) patients received primary or adjuvant radiosurgery or radiation therapy., Conclusion: Approximately 70% of patients with skull base tumors experienced recovery in oculomotor function post-treatment. The duration before treatment and the type of tumor were significantly associated with the oculomotor outcome. These findings aid neuro-ophthalmologists in predicting oculomotor outcomes for patients with skull base tumors, guiding management strategies for oculomotor dysfunction., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Moon, Lee. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: When operating near cranial motor nerves, transient postoperative weakness of target muscles lasting weeks to months is often observed. As nerves are typically intact at a procedure's completion, paresis is hypothesized to result from a combination of neurapraxia and axonotmesis. As both neurapraxia and axonotmesis involve Schwann cell injury and require remyelination, we developed an in vitro RSC96 Schwann cell model of injury using hydrogen peroxide (H 2 O 2 ) to induce oxidative stress and investigated the efficacy of candidate therapeutic agents to promote RSC96 viability. As a first step in developing a long-term local administration strategy, the most promising of these agents was incorporated into sustained-release microparticles and investigated for bioactivity using this assay., Methods: The concentration of H 2 O 2 which reduced viability by 50% was determined to establish a standard for inducing oxidative stress in RSC96 cultures. Fresh cultures were then co-dosed with H 2 O 2 and the potential therapeutics melatonin, N-acetylcysteine, resveratrol, and 4-aminopyridine. Schwann cell viability was evaluated and the most efficacious agent, N-acetylcysteine, was encapsulated into microparticles. Eluted samples of N-acetylcysteine from microparticles was evaluated for retained bioactivity., Results: 100 µM N-acetylcysteine improved the viability of Schwann cells dosed with H 2 O 2 . 100 µM Microparticle-eluted N-acetylcysteine also enhanced Schwann cell viability., Conclusion: We developed a Schwann cell culture model of iatrogenic nerve injury and used this to identify N-acetylcysteine as an agent to promote recovery. N-acetylcysteine was packaged into microparticles and demonstrated promise as a locally administrable agent to reduce oxidative stress in Schwann cells., (© 2024. The Author(s).)

Background: Arachnoid cysts (ACs) are benign lesions typically believed to not cause neurologic defects in the adult population and are most often found incidentally on imaging. We describe 2 patients with ACs potentially leading to isolated cranial nerve (CN) dysfunction., Methods: We describe 2 patients, 1 with a fourth nerve palsy and the other with a sixth nerve palsy found to have ACs on MRI brain imaging in locations that potentially caused a compressive CN palsy. We review previous literature of ACs presenting with CN III, IV, or VI palsy., Results: Patient 1 was a 62-year-old man who presented with a 22-year history of diplopia with strabismus examination consistent with a congenital CN IV palsy. Despite multiple surgeries, his CN IV palsy insidiously worsened. An AC in the posterior fossa with mass effect on the quadrigeminal plate and asymmetric atrophy of the right superior oblique was identified on imaging. Patient 2 was an 80-year-old man who presented with an 18-year history of diplopia and was found to have a left esotropia and abduction deficit consistent with complete CN VI palsy. An AC in the left cavernous sinus was identified on imaging. He underwent strabismus surgery with satisfactory resolution of diplopia. We identified a total of 18 previously published cases: 8 reports of CN III palsy, 4 reports of CN IV palsy, and 6 reports of CN VI palsy. Patient ages ranged from 1 to 67 with a median of 34.5. In 16/18 (89%) cases, the diagnosis of ACs was made within 1 year of symptom onset. Surgical removal of the AC was successful in resolving diplopia in 7/12 (58%) cases. In no case was strabismus surgery performed as primary treatment., Conclusions: Although ACs are typically congenital asymptomatic lesions, we present a case series of 2 patients with ACs in anatomic locations that potentially caused chronic, progressive, isolated CN palsies leading to strabismus. Our literature review revealed that most published cases detailing this clinical scenario resulted in neurosurgical fenestration of ACs with mixed results. Our cases represent 2 patients with AC-associated CN palsy treated with strabismus surgery., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Background and Objective: Understanding whether cranial nerve palsy (CNP) acts as an independent risk factor for kidney cancer could have important implications for patient care, early detection, and potentially the development of preventive strategies for this type of cancer in individuals with CNP. This study aimed to examine the risk of kidney cancer following the onset of ocular motor CNP and assess whether CNP could be considered an independent risk factor for kidney cancer. Materials and Methods: A population-based cohort study was conducted using data from the National Sample Cohort (NSC) database of Korea's National Health Insurance Service which was collected from 2010 to 2017. Follow-up was until kidney cancer development, death, or 31 December 2018. Cox proportional hazard regression analysis was performed to determine hazard ratios (HRs) for kidney cancer according to CNP status. Participants aged 20 years or more diagnosed with CNP from 2010 to 2017 were included. Exclusions comprised individuals with specific pre-existing conditions, inability to match a control group, and missing data, among others. CNP patients were age-sex matched in a 1:5 ratio with control cases. The primary outcome was incidence of kidney cancer during the follow-up period. Results: This study comprised 118,686 participants: 19,781 in the CNP group, and 98,905 in the control group. Compared to the control group, participants with CNP had a higher risk of kidney cancer (adjusted HR in model 4, 1.599 [95% CI, 1.116-2.29]). After a 3-year lag period, the CNP group had a significantly higher risk (adjusted HR in model 4, 1.987 [95% CI, 1.252-3.154]). Conclusions: Ocular motor CNP may be an independent risk factor for kidney cancer, as indicated by a higher incidence of kidney cancer in CNP patients. Further research is needed to elucidate the underlying mechanisms and explore potential preventive measures for kidney cancer in patients with ocular motor CNP.

Purpose of Review: To provide a summary of the visual manifestations and cranial neuropathies seen in Lyme disease., Recent Findings: Lyme facial palsy remains the most common manifestation of Lyme neuroborreliosis. Recent investigations show likely evidence of vagal involvement in Lyme disease., Summary: The literature on Lyme neuroborreliosis continues to evolve. Lyme disease can affect nearly any cranial nerve in addition to causing various headache syndromes. The most common manifestation is Lyme disease facial palsy, occurring in up to 5-10% of patients with documented Lyme disease. Headache syndromes are common in the context of facial palsy but can occur in isolation, and more specific headache syndromes including trigeminal and geniculate neuralgias can occur rarely. Signs and symptoms indicative of vestibulocochlear nerve involvement are relatively common, although it could be that these represent other vestibular involvement rather than a specific cranial neuropathy. Optic neuritis is a controversial entity within Lyme disease and is likely overdiagnosed, but convincing cases do exist. Physicians who see any cranial neuropathy, including optic neuritis, in an endemic area can consider Lyme disease as a possible cause., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

The typical presentation of multifocal motor neuropathy (MMN) is progressive asymmetric limb weakness. Cranial neuropathy is rare. We report a 28-year-old woman with cranial and bulbar palsies but with typical electrophysiological features of MMN by multifocal motor conduction blocks and serological markers of anti-ganglioside GM1 antibodies. The previous consensus on the treatment of MMN is intravenous immunoglobulins, but our patient responded to oral steroids and had clinical and electrophysiological improvement under continuous low-dose prednisolone treatment. In summary, MMN is a treatable chronic inflammatory disease of peripheral nerves. Cranial neuropathies can be its initial presentations. Electromyography studies are crucial for MMN diagnosis and helpful in monitoring disease activity and treatment responses. Although the previous guideline did not suggest using steroids for MMN, with careful patient selection, low-dose oral steroids can be an effective treatment in patients with relatively minor symptoms. Keywords: Multifocal motor neuropathy, conduction block, bulbar palsy, cranial nerve, cranial neuropathy.

Purpose : Minimally invasive and surgical spine procedures are commonplace with various risks and complications. Cranial nerve palsies, however, are infrequently encountered, particularly after procedures such as lumbar punctures, epidural anesthesia, or intrathecal injections, and are understandably worrisome for clinicians and patients as they may be interpreted as secondary to a sinister etiology. However, a less commonly considered source is a pneumocephalus which may, in rare cases, abut cranial nerves and cause a palsy as a benign and often self-resolving complication. Here, we present the case of a patient who underwent an intrathecal methotrexate infusion for newly diagnosed non-Hodgkin's T -cell lymphoma and subsequently developed an abducens nerve palsy due to pneumocephalus. We highlight the utility of various imaging modalities, treatment options, and review current literature on spinal procedures resulting in cranial nerve palsies attributable to pneumocephalus presenting as malignant etiologies., Competing Interests: Declaration of conflicting interestsThe authors have no conflicts of interest to declare that are relevant to the content of this article.

Spinal anesthesia is one of the most frequently performed regional anesthesia techniques for a variety of surgeries world-wide. Cranial nerve palsy is a rarely reported complication of central neuraxial block. The etiology varies; however, it is most often associated with nerve compression or traction due to intracranial hypotension. In October 2023, we searched PubMed and Google Scholar databases for English-language articles published between 1952 and 2023. The following search terms were used in the search strategy: olfactory, optic, oculomotor, trochlear, trigeminal, abducens, facial, vestibulocochlear, glossopharyngeal, vagus, accessory, hypoglossal nerve palsies, and epidural, spinal anesthesia, or dural puncture. The search was limited to humans and case reports written in English. We analyzed 89 articles and case reports in this review. In this article, a review of 105 cases published so far in the literature is presented. Cranial nerve palsies were more common in obstetric and gynecological cases. The 6th cranial nerve palsy was reported most frequently. Paralysis of more than one cranial nerve may develop simultaneously and may be bilateral or unilateral. In general, unilateral paralysis has been observed. The most common finding in 3rd, 4th, and 6th cranial nerve palsies was diplopia. In 8th cranial nerve palsy, hearing loss was the most observed symptom. PDPH is mostly associated with cranial palsies in most cases. It was observed that early recognition of patients with symptoms and utilization of diagnostic methods were effective in treatment. The most common cranial nerve injuries following spinal and epidural anesthesia and dural puncture are 6th and 3rd cranial nerve palsies. Symptoms are believed to occur mainly due to variations in cerebrospinal fluid (CSF) pressure. It is recommended to design treatment plans based on the mechanism.

Background: Current management guidelines for the treatment of carotid stenosis are controversial. We performed this meta-analysis to evaluate the perioperative safety of carotid artery stenting (CAS) and endarterectomy., Methods: We systematically searched EMBASE, PubMed, Web of Science, and the Cochrane Library from inception to November 10, 2022, for randomized controlled trials that compared CAS with carotid endarterectomy (CEA) among patients with carotid stenosis. The analyzed outcomes mainly included stroke, death, myocardial infarction (MI), cranial nerve palsy, the cumulative incidence of mortality, stroke, or MI and the cumulative incidence of death or stroke in the perioperative periods. The risk ratio (RR) and 95% confidence interval (95% CI) were calculated and pooled. Subgroup analyses were based on whether patients were symptomatic or asymptomatic. We assessed the certainty of evidence using the Grading of Recommendations Assessment, Development and Evaluation framework., Results: Seventeen randomized controlled trials with 12,277 participants (6514 and 5763 in the CAS and CEA groups, respectively) were included. Pooled analysis demonstrated that compared with CEA, CAS was associated with decreased risks of perioperative MI (RR = 0.47, 95% CI = 0.29∼0.77) and perioperative cranial nerve palsy (RR = 0.02, 95% CI = 0.01∼0.06) but higher risks of perioperative stroke (RR = 1.48, 95% CI = 1.18∼1.87) and cumulative incidence of death or stroke (RR = 1.52, 95% CI = 1.20∼1.93)., Conclusions: The perioperative safety was equivalent between CAS and CEA. However, CEA may be preferred when considering both procedural safety and long-term efficacy in preventing recurrent stroke., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Microvascular decompression is a widely accepted surgical treatment for compressive cranial nerve pathologies such as trigeminal neuralgia, hemifacial spasm, glossopharyngeal neuralgia, and other craniofacial pain syndromes. Endoscopy has risen as a safe and effective minimally invasive tool to optimize microvascular decompression. Endoscopy offers improved visualization, minimizes retraction, and allows for smaller surgical openings compared to traditional microscopic approaches. There are several reports of improved neuralgia outcomes and reduced post-operative complications after endoscopic microvascular decompression. In skilled surgical hands, endoscopy is an excellent option for microvascular decompression as stand-alone tool or adjunct to the microscope. An overview of the history, operative considerations, and techniques is provided in this chapter., (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

Background: In patients with traumatic head injuries, the percentage of cranial nerve injuries (CNI) range from4.3 to 17.6% in which majority are isolated CNI[1-5].In present literature, moderate to severe types of head injuries are often studied which may result in a lack of representation and description of CNI associated with minor head injuries (MHI). Alongside this peculiar case of a traumatic cavernous sinus syndrome (CSS) that is non-thrombotic and non-fistulous in nature, this paper aims to analyse traumatic CNI in non-severe head injuries and the surrounding literature., Case Report: A 65-year-old man who had sustained a minor head injury was found to have CNI of III, IV and VI.Brain imaging showed scattered traumatic subarachnoid haemorrhage and a non-displaced right zygomatic arch fracture. Despite the short course of high dose dexamethasone, he showed only partial recovery of his CNI after one year., Conclusion: We present a case of traumatic CSS likely secondary to tractional injury from a MHI. Injury to the extraocular nerves wasfound to be one of the more commonly observed combination of CNI from the literature review conducted. In patients with MHI, multiple CNI is less common. Hence, consideration should be given to work upfor secondary causes such as tumours. There is presently no known clear identifiable pattern of CNI associated with MHI. CT brain findings of skull base fractures and early onset of cranial nerve palsies are generally associated with worse outcomes. More remains to be studied about tractional CNI in non-severe head injuries., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Although flow diversion plays a pivotal role in treating internal carotid artery aneurysms presenting with cranial neuropathy, predictors of symptom improvement have not been established., Objective: To investigate improvement of symptoms after flow diversion treatment in patients with internal carotid artery aneurysms causing cranial neuropathy, with sufficient follow-up period. Additionally, to examine factors associated with improvement of symptoms., Methods: This retrospective multicenter study examined patients with unruptured internal carotid artery aneurysms presenting with cranial neuropathy who were treated using flow diversion and followed up for at least 12 months. Study outcomes were transient worsening of symptoms and symptom status 12 months after treatment. Patient and aneurysm characteristics were statistically analyzed., Results: Seventy-seven patients were included. Data needed for outcome analysis were available for 66 patients. At the 1-, 3-, 6-, 12-month, and last follow-ups, the proportion of patients with resolved or improved symptoms was 26% (20/77), 51% (39/77), 74% (57/77), 83% (64/77), and 79%(62/77), respectively. Symptom onset-to-treatment time <6 months (OR=24.2; 95% CI 3.09 to 188.84; p=0.002) and aneurysmal regression (OR=23.1; 95% CI 1.97 to 271.75; p=0.012) were significantly associated with symptom improvement. Transient symptom worsening and worse symptoms at 12 months occurred in 19/77 (25%) and 2/77 (3%) patients, respectively., Conclusions: The rate of cranial neuropathy symptom improvement after flow diversion increased over the first 12 months after treatment, but not thereafter. Treatment within 6 months of symptom onset and aneurysmal regression were predictors of symptom improvement., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY. Published by BMJ.)

Objective: This study aimed to clarify the symptoms of pituitary or parasellar tumor onset with cranial nerve palsy (CNP) and to improve our knowledge of this rare symptom and its most appropriate treatment., Methods: Among 1281 patients with pituitary or parasellar tumors surgically treated from 2003 to 2020, 30 cases (2.34%; 15 men and 15 women; mean age: 55.6 years, range: 6-83 years) first presenting with CNP were reviewed to evaluate the neurological symptoms, histological diagnosis, interval from onset to surgery, and time before complete CNP recovery., Results: Pathological diagnoses comprised 17 pituitary adenomas, including 10 pituitary apoplexies and 4 adrenocorticotropic hormone-positive adenomas, and 13 other tumors, including 3 chordomas, 2 xanthogranulomas, 2 malignant lymphomas, 2 metastatic tumors, 1 Rathke cleft cyst, 1 plasmacytoma, 1 craniopharyngioma, and 1 neuroendocrine carcinoma. The mechanisms causing CNP were pituitary apoplexy (n = 10), cranial nerve compression or involvement (n = 17), and inflammatory changes (n = 9). As the first manifestation, 20 (66.7%) patients presented with oculomotor nerve palsy, 2 (6.7%) with trochlear nerve palsy, and 13 (43.3%) with abducens nerve palsy. Full recovery of CNP was obtained in 25 patients (83.3%) after surgery alone and in 2 patients (6.7%) after adjuvant therapy. Early surgery provided no significant difference in full recovery rates although it reduced the time to reach full recovery., Conclusions: It is critical to determine the mechanisms of CNP and intervene surgically to improve symptoms, shorten the duration of the disorder, prevent relapses, and obtain the correct pathological diagnosis to select the proper adjuvant therapy., (Copyright © 2023 Elsevier Inc. All rights reserved.)

We report a case of a previously healthy man in his 40s who presented with mild SARS-CoV-2 infection (COVID-19) concomitant with acute onset of left third cranial nerve palsy with restricted supraduction, adduction and infraduction. Our patient did not present any history of hypertension, hyperlipidaemia, diabetes mellitus or smoking. The patient recovered spontaneously without any antiviral treatment. To our knowledge, this is the second report of third cranial nerve palsy spontaneously resolved without any risk factors of vascular disease, specific image findings, nor any possible causes other than COVID-19. In addition, we reviewed 10 other cases of third cranial nerve palsy associated with COVID-19, which suggested that the aetiology varies greatly. As a clinician, it is important to recognise COVID-19 as a differential diagnosis for third cranial nerve palsy. Finally, we aimed to encapsulate the aetiologies and the prognosis of the third cranial nerve palsy associated with COVID-19., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

The signs of lower cranial nerve palsies are rare and are often caused by tumors. A 49-year-old woman was admitted to our hospital with progressive right-sided atrophy of the tongue, sternocleidomastoid and trapezius, dysarthria, and dysphagia for 3 years. Brain magnetic resonance imaging revealed a circular lesion adjacent to the lower cranial nerves. Cerebral angiography confirmed that the lesion was an unruptured aneurysm in the C1 segment of the right internal carotid artery. After endovascular treatment, the symptoms of this patient had partially improved., (Copyright © 2023 Elsevier Inc. All rights reserved.)

We present varicella-zoster virus (VZV) infection with concomitant lower cranial polyneuropathy in the absence of meningeal symptoms. Physical examination showed involvement of cranial nerves IX and X in Case 1 and of cranial nerves IX, X, and XI in Case 2. Cerebrospinal fluid (CSF) analysis revealed mild lymphocytic pleocytosis, normal protein levels, and absence of VZV-DNA based on polymerase chain reaction (PCR) analysis. Serum anti-VZV antibody testing showed positive results in both cases, which confirmed the diagnosis of VZV infection. VZV infection accompanied by lower cranial polyneuropathy is rare; therefore, it is important to consider VZV reactivation as an etiopathogenetic contributor to pharyngeal palsy and hoarseness. We emphasize the importance of serological analysis for precise diagnosis in VZV infection with multiple lower cranial nerve palsies because the VZV-DNA PCR test may show negative results in patients without meningitis symptoms or in those with normal CSF protein levels.

Pituitary adenomas are benign brain tumors that comprise 10%-20% of all central nervous system neoplasms. In recent years, stereotactic radiosurgery (SRS) has emerged as a highly effective treatment option in the management of functioning and nonfunctioning adenomas. It is associated with tumor control rates frequently ranging from 80% to 90% in published reports. While permanent morbidity is uncommon, potential side effects include endocrine dysfunction, visual field deficits, and cranial nerve neuropathies. In patients where single fraction SRS would pose an unacceptable risk (e.g. large lesion size or close proximity to the optic apparatus), hypofractionated SRS delivered in 1-5 fractions is a potential treatment option; however, available data are limited. A comprehensive literature search of PubMed/MEDLINE, CINAHL, Embase, and the Cochrane Library was conducted to identify articles reporting on the use of SRS in functioning and nonfunctioning pituitary adenomas., Competing Interests: None

Objective: Tympanojugular paragangliomas (TJPs) are slow-growing tumors arising within the middle ear or jugular foramen. The development of modified skull base approaches and the increasing use of stereotactic radiosurgery have provided more modern techniques in the management of TJPs. Several factors dictating approach selection, and resulting clinical outcomes have been inconsistently described., Methods: A systematic review of the literature describing modern management of complex TJPs was performed and summarized. A random-effects meta-analysis was performed to describe the rate of tumor control, complications, and symptom improvement in patients undergoing radiosurgery or surgical resection., Results: Nineteen studies were identified with a total of 852 TJP patients. A minority (153 patients) underwent radiosurgery while 699 underwent surgery. On meta-analysis, there was a 3.5% (95% confidence interval [CI]: 0.5%-6.4%) tumor growth rate following radiosurgery and 3.9% (95% confidence interval [CI]: 1.8%-6.0%) recurrence rate in surgical resection, with no significant moderator effect between the 2 groups (P = 0.9046). Complication rate for radiosurgery was 7.6% (95% CI: 2.8%-12.4%), differing significantly from surgical complication rates of 29.6% (95% CI: 17.1-42.0%, P = 0.0418)., Conclusions: Stereotactic radiosurgery and surgical resection for TJPs have similar rates of tumor recurrence. Radiation is associated with less risk and lower morbidity, yet there is comparably modest reduction of the tumor size. In sum, the data suggest that radiosurgery is a reasonable management option for patients with minimal symptoms who are high risk for surgery. Microsurgical resection should be reserved for patients with lower cranial neuropathies or those who have failed radiation treatment., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Various neurological manifestations involving the central and peripheral nervous system have been reported in association with COVID-19. Most common associations reported are encephalopathy, headache, ischemic, hemorrhagic stroke and transient ischemic attack, Miller Fisher syndrome, cranial neuropathies and Guillain-Barre syndrome. Of the cranial neuropathies, anosmia, and dysgeusia are the most common reported symptoms. This is a case of COVID-19 with ipsilateral fifth and seventh cranial nerve involvement with complete resolution of symptoms over a period of 3 weeks. The neurological symptoms started within 5 days of respiratory symptoms. We conclude that isolated cranial neuropathies can be the manifestations of SARS-CoV-2 infection.

Background: Hypertrophic pachymeningitis (HPM) is a unique disorder characterized by thickening and fibrosis of the dura mater. Clinically it presents with headache, cranial nerve palsies, and other focal neurological deficits. Two forms exist, one is primary, where all other causes have been excluded and the other is secondary where an identifiable cause exists. It is important to recognize these secondary causes as treatment depends on the etiology., Objective: To elucidate the various characteristics of HPM. To delineate clinical-radiological features that help differentiate secondary from primary causes and to understand treatment response and disease outcomes of HPM., Methods: This retrospective observational study included 33 patients who presented with radiological diagnosis of HPM from January 2014 to July 2019. Spontaneous intracranial hypotension patients were excluded. All patients were extensively evaluated for secondary causes and treatment outcomes were analyzed on follow-up., Results and Conclusions: Secondary causes of HPM were present in 48% cases. The clue for primary causes is an associated Tolosa-Hunt syndrome. Secondary causes in our series are immunological, infection, and malignancy. Clues to differentiate primary from these secondary causes are clinical like myelopathy, seizures, poor response to immunosuppression; radiological like hypertrophic cranial nerves, infarcts, bony erosion, and leptomeningeal involvement. There are case reports in literature but large Indian studies are lacking. This manuscript presents a large cohort of cases with HPM, which helps differentiate primary from secondary causes, as management and prognosis depend on etiology. An algorithm depicting the approach to the management of HPM has been presented., Competing Interests: None

Introduction: There are various etiologies for isolated third, fourth and sixth cranial nerve palsies. The most common etiology in adults aged 50 years and older with vascular risk factors such as diabetes, hyperlipidemia, and hyperlipidemia, is microvascular ischemia. The role of early neuroimaging in older patients with vascular risk factors presenting with acute isolated ocular motor nerve palsy is controversial., Aims: We present six cases of patients aged 50 years and older, with vascular risk factors, who suffered from acute isolated paralysis of the sixth nerve. All cases were diagnosed with a non-microvascular ischemia etiology., Results: The identified etiologies included petroclival meningioma with involvement of the cavernous sinus, metastatic hepatoma, plasmacytoma, venous thrombosis, arterial-venous malformation and aneurysm., Conclusions: Despite the high cost and presumed low yield, early neuroimaging can change the clinical management in certain cases., Discussion: Previous studies have found the yield of early neuroimaging in older vasculopathic patients with acute 4th or 6th cranial nerve palsies to be 1-15%. In spite of these reports, our cases demonstrate the need for early onset imaging in these cases.

Background: We report a rare case of ipsilateral multiple cranial neuropathy and ipsilateral lymphadenopathy following mRNA-COVID-19 vaccination., Case Presentation: A 41-year-old male visited our emergency room complaining of dysphagia and hoarseness that started a week after receiving COVID19 mRNA vaccination (in his right arm). During his hospitalization, he also complained of right side hearing loss and diplopia. Neurological examination depicted a right IV nerve palsy, ipsilateral facial paresthesia and peripheral facial paresis. Otorinolaryngological examination revealed right vocal cord paralysis. A brain magnetic resonance imaging showed enhancement of the right VII and VIII cranial nerves in the auditory canal. The lumbar puncture revealed increased protein concentration and lymphocytic pleocytosis in the cerebrospinal fluid (CSF). Additionally, a neck computed tomography (CT) scan showed a swollen right supraclavicular lymph node. We hypothesize that the ipsilateral cranial neuropathies of IV, VI, VII, VIII and X, associated with cervical lymphadenopathy, was possible caused by a post-vaccination immune-mediated reaction. The patient was treated with a 5-day course of intravenous methylprednisolone (1000 mg/day), and a gradual improvement was observed., Conclusions: Similarly, to other vaccines, it is possibly that also mRNA vaccines may act as triggers of non-specific autoimmune neurological syndromes., (© 2022. The Author(s).)

Early disseminated Lyme disease can involve the peripheral or central nervous system, but with early diagnosis and treatment, prognosis for full recovery is excellent. The typical clinical presentations of neuroborreliosis are highlighted, and an approach to diagnosis and treatment is described., Competing Interests: Disclosure There are no commercial, or financial conflicts of interest, or funding sources for the authors., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: Multiple cranial neuropathies (MCN) is an entity frequently seen in clinical practice but there is a lack of studies published about this entity, with most of them based on case reports and small case series., Objective: The aim of this study is to describe the clinical involvement of different cranial nerves, the location within the central or peripheral nervous system and the diagnosis in a group of patients with MCN managed in one hospital in Bogotá-Colombia., Methodology: A case series study was conducted using the electronic clinical records of a teaching hospital in Bogota-Colombia. Clinical data were collected from patients aged ≥18 with a clinical diagnosis of MCN between 2015 and July 2021., Results: The cranial nerves most commonly affected were III and VII, with the most prevalent combinations being III-IV, III-VI, and V-VII. Among etiologies, the most frequently found were autoimmune, vascular and neoplastic and most common locations included peripheral nerves, neuromuscular junction, cavernous sinus and lateral medulla., Conclusions: The differential diagnosis of MCN is broad, but clinical clues may aid in identifying the underlying etiology. According to our results, MG was the most frequent etiology, so it should be considered in any patient with a clinical diagnosis of MCN associated with fatigability., (Copyright © 2022 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: Multiple cranial neuropathies carry a wide range of differential diagnoses, and when combined with cerebrospinal fluid monocytosis they often suggest an infective etiology. Reactivation of varicella zoster virus has been associated with a wide range of neurological complications. Among the cranial nerves, the upper cranial nerves (trigeminal and facial nerves) are more commonly affected; there have been some reports of lower cranial polyneuropathies resulting from varicella zoster virus reactivation. However, polyneuropathy involving both the cranial and cervical nerves is rarely reported., Case Presentation: This report highlights the case of a 64-year-old Chinese man presenting with an acute, severe dysphagia and dysphonia secondary to herpes zoster-associated progressive polyneuropathy involving the lower cranial and upper cervical nerves, without any mucocutaneous manifestations., Conclusions: To our knowledge, this is the first case of varicella zoster virus-associated cranial and cervical polyneuropathy in the literature. The report also highlights the poor sensitivity of varicella zoster virus DNA detection by polymerase chain reaction in the cerebrospinal fluid, and proposes that serology be routinely performed in such polymerase chain reaction-negative cases without a clear diagnosis., (© 2022. The Author(s).)

Background and Purpose: Skull base metastases are a severe complication of various malignant tumors. If cranial nerves are involved even small lesions can cause significant symptoms. Specific clinical characteristics like neurological symptoms, associated primary tumors, prognosis and optimal treatment are poorly defined and are systematically described in this article., Methods: In a monocentric retrospective study patients with skull base metastases and cranial nerve deficits who received treatment between 2006 and 2018 were analyzed concerning clinical characteristics at initial diagnosis, treatment and course of the disease., Results: In this study 45 patients with skull base metastases and cranial nerve deficits were included. The most frequent primary tumors were prostate cancer (27%), breast cancer (22%) and multiple myeloma (16%). The most involved cranial nerves were trigeminal nerve (42%), oculomomotor nerve (33%) and facial nerve (27%). Of the patients 84% had additional bone metastases outside the skull base. Dural infiltration or meningeal carcinomatosis were each observed in 13% of the patients. After radiotherapy cranial nerve deficits remained stable in 61% of all cases and in 22% symptoms improved. Median overall survival from treatment was 8 months (range 0.4-51 months). Patients with dose-escalated radiotherapy appeared to live longer (16.4 months vs. 4.7 months). This effect persisted in a multivariate analysis including the Karnofsky index, number of metastases, primary tumor and radiation dose (HR 0.37, p = 0.02)., Conclusion: Skull base metastases with cranial nerve deficits are complex diseases with poor prognosis. Precise diagnosis and treatment are required. Further research is needed to improve treatment., (© 2022. The Author(s).)

Background: Craniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. We systematically reviewed the literature on CVJ schwannomas., Methods: PubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA statement to include studies reporting CVJ schwannomas. Clinical features, management, and outcomes were analyzed., Results: We collected 353 patients from 101 included articles. Presenting symptoms were mostly neck pain (30.3%) and headache (26.3%), with most cranial neuropathies involving the XII (31.2%) and X (24.4%) nerves. Most tumors originated from C2 (30.9%) and XII (29.4%) nerves, being extracranial (45.1%) and intradural-extradural (44.2%). Erosion of C1-C2 vertebrae (37.1%), the hypoglossal canal (28.3%), and/or jugular foramen (20.1%) were noted. All tumors were operated, preferably with the retrosigmoid approach (36.5%), with the far-lateral approach (29.7%) or with the posterior approach and cervical laminectomy (26.9%), far-lateral approaches (14.2%), or suboccipital craniotomy with concurrent cervical laminectomy (14.2%). Complete tumor resection was obtained most frequently (61.5%). Adjuvant post-surgery stereotactic radiosurgery was delivered in 5.9% patients. Median follow-up was 27 months (range, 12-252). Symptom improvement was noted in 88.1% of cases, and cranial neuropathies showed improvement in 10.2%. Post-surgical complications occurred in 83 patients (23.5%), mostly dysphagia (7.4%), new cranial neuropathies (6.2%), and cerebrospinal fluid leak (5.9%). A total of 16 patients (4.5%) had tumor recurrence and 7 died (2%), with median overall survival of 2.7 months (range, 0.1-252)., Conclusions: Microsurgical resection is safe and effective for CVJ schwannomas. Data on SRS efficacy and indications are still lacking, and its role deserves further evaluation.

Skull base metastatic tumors are rare. Breast cancer in particular can cause bone metastases after a long period of time. A 70-year-old woman presented with multiple cranial nerve palsy. Magnetic resonance imaging revealed a lesion that extended from the orbit to the base of the skull, and the patient was referred to our department. Ophthalmological evaluation showed left visual acuity impairment, left oculomotor nerve palsy, and left trochlear nerve palsy. Endoscopic biopsy performed 5 years after the completion of breast cancer treatment revealed skull base metastases. In unilateral multiple cranial nerve palsy, the possibility of skull base metastases should be considered.

A 73-year-old man developed delayed-onset multiple cranial neuropathies of cranial nerves V, VII and VIII, and segmental paresis in the ipsilateral upper extremity related to the C4 to Th1 segment, after all skin lesions with varicella zoster (VZV) on the left neck of the C3-4 dermatome had dried and crusted over. On admission, cerebrospinal fluid (CSF) revealed pleocytosis (all mononuclear cells, 12/μl). Treatment was started with intravenous acyclovir (10 mg/kg, every 8 h for 14 days) and methylprednisolone (1,000 mg/day for 3 days). Four days after starting treatment, left segmental paresis was improved, but the multiple cranial neuropathies persisted. Oral prednisolone (0.5 mg/kg/day) was administered for 5 days, then tapered off. All neurological symptoms had disappeared by hospital day 23. Of particular interest was the discrepancy between skin regions affected by VZV (C3-4) and the regions of cranial neuropathy (cranial nerves V, VII, and VIII) and muscle weakness innervated by C4-Th1. Although CSF was negative for VZV DNA according to PCR testing, the antibody index for VZV was elevated. This suggests intrathecal synthesis of VZV antibodies and supports the diagnosis of VZV meningitis. Also, all cranial nerves involved in this case were reported to have the cranial nerve ganglia where VZV could have established latency and been reactivated. This suggests concurrent reactivation on each cranial nerve ganglia without cutaneous lesions, as zoster sine herpete. In addition, anastomoses among the upper cervical nerves, which are found in some patients, may have contributed to this condition. These mechanisms underlie various neurological symptoms associated with VZV infection.

Primary ductal adenocarcinoma of the lacrimal gland is a rare, aggressive malignancy that clinically and histologically resembles salivary duct carcinoma. Similar to other malignant epithelial lacrimal gland tumors, ductal adenocarcinoma typically presents with unilateral proptosis, pain, upper eyelid swelling, palpable mass, diplopia, ptosis, and blurred or decreased vision. Rarely, primary malignant epithelial lacrimal gland tumors may first present with multiple cranial neuropathies due to occult spread to the cavernous sinus, as in this case. With such a vast differential diagnosis, a practical yet systematic approach to multiple cranial neuropathies, as guided by clinical history, exam, and neuroimaging, allows for a more targeted diagnostic evaluation, especially when multiple diagnostic tests and interventions return unrevealing. A repeat biopsy or complete excision of the lacrimal gland may be necessary to yield the correct diagnosis., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)